Von Hippel-Lindau: implications in development and disease—response

The authors would like to thank Park et al. for their insightful editorial commentary on our recently published article titled “Von Hippel-Lindau Acts as a Metabolic Switch Controlling Nephron Progenitor Differentiation” (1). The commentary provides an excellent overview of the VHL/HIF axis and its role in cellular oxygen sensing. The editorial authors correctly imply that our study revealed a novel role for VHL in kidney development such that nephron progenitors are required to undergo a metabolic switching event. This reprogramming from glycolysis to mitochondrial respiration was also found to be critical for adequate nephron formation (1).